Crypt cell carcinoma

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Crypt cell carcinoma
Diagnosis in short

Crypt cell carcinoma. H&E stain.

LM small clusters of cells with stippled chromatin and a goblet cell-like appearance
LM DDx signet ring cell carcinoma, appendiceal neuroendocrine tumour, poorly differentiated adenocarcinoma
Stains alcian blue +ve, PASD +ve, mucicarmine +ve
IHC synaptophysin +ve, chromogranin +ve, S-100 +ve, CK20 +ve
Gross usu. no mass apparent
Site vermiform appendix, elsewhere in the GI tract

Clinical history "acute appendicitis"
Prevalence rare
Prognosis moderate
Clin. DDx acute appendicitis, other appendiceal tumours, other abdominal pathology

Crypt cell carcinoma, also known as goblet cell carcinoid[1][2] and neuroendocrine tumour with goblet cell differentiation, is a rare malignant tumour that is typically seen in the vermiform appendix.

General

  • Rare appendiceal tumour that typically has an aggressive course vis-a-vis other appendiceal carcinoids.[1]
  • Mixed (biphasic) tumour with endocrine and exocrine features.
  • Usually presents as acute appendicitis.[2]
    • Less common presentations: appendiceal mass, pain.
  • Five year survival in one series: 60-85%.[2]

Gross

  • Typically no mass is apparent at gross.[2]

Note:

Microscopic

Features:[2]

  • Mixed neuroendocrine-nonneuroendocrine tumour;[3] features of both carcinoid and adenocarcinoma.[2]
    • Archictecture: cells arranged in nests or clusters without a lumen.
    • Location: deep to the intestinal crypts (crypts of Lieberkühn); usually do not involve the mucosa.
    • Cytoplasm distended with mucin.
    • DNA: crescentic nucleus (similar to in signet ring cells).
      • +/-Multi-nucleation.
      • +/-High mitotic rate.
      • Usually minimal nuclear atypia.

DDx:

Images

Case 1

Case 2

Stains

IHC

  • Classic neuroendocrine markers:
    • Synaptophysin +ve.
    • Chromogranin +ve.
  • S-100 +ve.
  • NSE +ve.
  • Serotonin +ve.

Keratins:

  • CEA +ve (membrane).

Notes:

  • Review of stains in Pahlavan and Kanthan.[2]

See also

References

  1. 1.0 1.1 van Eeden S, Offerhaus GJ, Hart AA, et al. (December 2007). "Goblet cell carcinoid of the appendix: a specific type of carcinoma". Histopathology 51 (6): 763–73. doi:10.1111/j.1365-2559.2007.02883.x. PMID 18042066.
  2. 2.0 2.1 2.2 2.3 2.4 2.5 2.6 Pahlavan, PS.; Kanthan, R. (Jun 2005). "Goblet cell carcinoid of the appendix.". World J Surg Oncol 3: 36. doi:10.1186/1477-7819-3-36. PMID 15967038.
  3. Volante M, Righi L, Asioli S, Bussolati G, Papotti M (August 2007). "Goblet cell carcinoids and other mixed neuroendocrine/nonneuroendocrine neoplasms". Virchows Arch. 451 Suppl 1: S61–9. doi:10.1007/s00428-007-0447-y. PMID 17684764.
  4. Pericleous, M.; Lumgair, H.; Baneke, A.; Morgan-Rowe, L.; E Caplin, M.; Luong, TV.; Thirlwell, C.; Gillmore, R. et al. (May 2012). "Appendiceal goblet cell carcinoid tumour: a case of unexpected lung metastasis.". Case Rep Oncol 5 (2): 332-8. doi:000339607. PMID 22933998.